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Tricuspid Atresia
Randy R. Richardson, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          TERMINOLOGY

          • Definitions

            • Congenital absence or agenesis of tricuspid valve & inlet portion of right ventricle (RV)

          IMAGING

          • General Features

            • Radiographic Findings

              • CT Findings

                • MR Findings

                  • Echocardiographic Findings

                    • Angiographic Findings

                      • Other Modality Findings

                        DIFFERENTIAL DIAGNOSIS

                          PATHOLOGY

                          • General Features

                            • Microscopic Features

                              CLINICAL ISSUES

                              • Presentation

                                • Demographics

                                  • Natural History & Prognosis

                                    • Treatment

                                      DIAGNOSTIC CHECKLIST

                                      • Consider

                                        • Image Interpretation Pearls

                                          Selected References

                                          1. Lee C et al: Tricuspid atresia with absent pulmonary valve and intact ventricular septum: successful bidirectional cavopulmonary anastomosis with complete exclusion of the right ventricle. Cardiol Young. 30(1):126-8, 2020
                                          2. Siddartha CR et al: Tricuspid atresia with truncus arteriosus: off-pump stage I palliation. World J Pediatr Congenit Heart Surg. 10(5):635-7, 2019
                                          3. Futuki A et al: Absent pulmonary valve syndrome with tricuspid atresia, ventricular septal defect, and aneurysmal dilated pulmonary artery: a case report of successful fontan completion. World J Pediatr Congenit Heart Surg. 9(1):101-4, 2018
                                          4. Enaba MM et al: Multidetector computed tomography (CT) in evaluation of congenital cyanotic heart diseases. Pol J Radiol. 82:645-59, 2017
                                          5. Kinoshita M et al: Energetic performance analysis of staged palliative surgery in tricuspid atresia using vector flow mapping. Cardiovasc Ultrasound. 15(1):27, 2017
                                          6. Alsoufi B et al: Influence of morphology and initial surgical strategy on survival of infants with tricuspid atresia. Ann Thorac Surg. 100(4):1403-9; discussion 1409-10, 2015
                                          7. Williams-Phillips S: Tricuspid atresia 18 years post Glenn: is Fontan necessary in all cases? West Indian Med J. 64(3), 2015
                                          8. Rathod RH et al: Myocardial fibrosis identified by cardiac magnetic resonance late gadolinium enhancement is associated with adverse ventricular mechanics and ventricular tachycardia late after Fontan operation. J Am Coll Cardiol. 55(16):1721-8, 2010
                                          9. Choi YH et al: CT findings in unilateral hepatopulmonary syndrome after the Fontan operation. Pediatr Radiol. 39(4):336-42, 2009
                                          10. Grosse-Wortmann L et al: Aortopulmonary collaterals after bidirectional cavopulmonary connection or Fontan completion: quantification with MRI. Circ Cardiovasc Imaging. 2(3):219-25, 2009
                                          11. Bhatti S et al: Persistent left superior vena cava (PLSVC) with anomalous left hepatic vein drainage into the right atrium: role of imaging and clinical relevance. Vasc Med. 12(4):319-24, 2007
                                          12. Sittiwangkul R et al: Outcomes of tricuspid atresia in the Fontan era. Ann Thorac Surg. 77(3):889-94, 2004
                                          13. Epstein M: Tricuspid atresia. In Allen HD et al: Heart Disease in Infants, Children & Adolescents. Philadelphia: Lippincott Williams & Wilkins. 1197-1215, 2001
                                          14. Hess J: Long-term problems after cavopulmonary anastomosis: diagnosis and management. Thorac Cardiovasc Surg. 49(2):98-100, 2001
                                          15. Freedom RM et al: The Fontan procedure: analysis of cohorts and late complications. Cardiol Young. 10(4):307-31, 2000
                                          Related Anatomy
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                                          Related Differential Diagnoses
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                                          References
                                          Tables

                                          Tables

                                          KEY FACTS

                                          • Terminology

                                            • Imaging

                                              • Top Differential Diagnoses

                                                • Clinical Issues

                                                  TERMINOLOGY

                                                  • Definitions

                                                    • Congenital absence or agenesis of tricuspid valve & inlet portion of right ventricle (RV)

                                                  IMAGING

                                                  • General Features

                                                    • Radiographic Findings

                                                      • CT Findings

                                                        • MR Findings

                                                          • Echocardiographic Findings

                                                            • Angiographic Findings

                                                              • Other Modality Findings

                                                                DIFFERENTIAL DIAGNOSIS

                                                                  PATHOLOGY

                                                                  • General Features

                                                                    • Microscopic Features

                                                                      CLINICAL ISSUES

                                                                      • Presentation

                                                                        • Demographics

                                                                          • Natural History & Prognosis

                                                                            • Treatment

                                                                              DIAGNOSTIC CHECKLIST

                                                                              • Consider

                                                                                • Image Interpretation Pearls

                                                                                  Selected References

                                                                                  1. Lee C et al: Tricuspid atresia with absent pulmonary valve and intact ventricular septum: successful bidirectional cavopulmonary anastomosis with complete exclusion of the right ventricle. Cardiol Young. 30(1):126-8, 2020
                                                                                  2. Siddartha CR et al: Tricuspid atresia with truncus arteriosus: off-pump stage I palliation. World J Pediatr Congenit Heart Surg. 10(5):635-7, 2019
                                                                                  3. Futuki A et al: Absent pulmonary valve syndrome with tricuspid atresia, ventricular septal defect, and aneurysmal dilated pulmonary artery: a case report of successful fontan completion. World J Pediatr Congenit Heart Surg. 9(1):101-4, 2018
                                                                                  4. Enaba MM et al: Multidetector computed tomography (CT) in evaluation of congenital cyanotic heart diseases. Pol J Radiol. 82:645-59, 2017
                                                                                  5. Kinoshita M et al: Energetic performance analysis of staged palliative surgery in tricuspid atresia using vector flow mapping. Cardiovasc Ultrasound. 15(1):27, 2017
                                                                                  6. Alsoufi B et al: Influence of morphology and initial surgical strategy on survival of infants with tricuspid atresia. Ann Thorac Surg. 100(4):1403-9; discussion 1409-10, 2015
                                                                                  7. Williams-Phillips S: Tricuspid atresia 18 years post Glenn: is Fontan necessary in all cases? West Indian Med J. 64(3), 2015
                                                                                  8. Rathod RH et al: Myocardial fibrosis identified by cardiac magnetic resonance late gadolinium enhancement is associated with adverse ventricular mechanics and ventricular tachycardia late after Fontan operation. J Am Coll Cardiol. 55(16):1721-8, 2010
                                                                                  9. Choi YH et al: CT findings in unilateral hepatopulmonary syndrome after the Fontan operation. Pediatr Radiol. 39(4):336-42, 2009
                                                                                  10. Grosse-Wortmann L et al: Aortopulmonary collaterals after bidirectional cavopulmonary connection or Fontan completion: quantification with MRI. Circ Cardiovasc Imaging. 2(3):219-25, 2009
                                                                                  11. Bhatti S et al: Persistent left superior vena cava (PLSVC) with anomalous left hepatic vein drainage into the right atrium: role of imaging and clinical relevance. Vasc Med. 12(4):319-24, 2007
                                                                                  12. Sittiwangkul R et al: Outcomes of tricuspid atresia in the Fontan era. Ann Thorac Surg. 77(3):889-94, 2004
                                                                                  13. Epstein M: Tricuspid atresia. In Allen HD et al: Heart Disease in Infants, Children & Adolescents. Philadelphia: Lippincott Williams & Wilkins. 1197-1215, 2001
                                                                                  14. Hess J: Long-term problems after cavopulmonary anastomosis: diagnosis and management. Thorac Cardiovasc Surg. 49(2):98-100, 2001
                                                                                  15. Freedom RM et al: The Fontan procedure: analysis of cohorts and late complications. Cardiol Young. 10(4):307-31, 2000